A set of identical female twins developed hematuria and a salt-losing state three days after birth. Evaluation at 10 months of age for failure to thrive revealed severe hypertension associated with interstitial nephritis and massive medial hypertrophy of the renal arterioles. Known causes of hypertension have been eliminated. To our knowledge, this clinical picture has not been previously described.Hypertensi on in the pediatrie age group is an unusual and challenging problem. By careful clin¬ ical investigation and the use of new diagnostic techniques, hyper¬ tension in most children may be as¬ cribed to one of the more than 25 causes listed by Rubin1 and Loggie.2 Occasionally, as in adults,3 the cause of hypertension in childhood remains unexplained. Recent reports4·5 de¬ scribing children with unexplained or "essential" hypertension have charMinneapolis 55455 (Dr. Bergstein). acterized these patients as being asymptomatic with diastolic blood pressures less than 120 mm Hg, as having a strong family history of hy¬ pertensive disease, and as having a benign course. This report documents the in¬ vestigation of identical female twins with a destructive form of hyper¬ tension occurring in infancy which, by its early onset, severity, absence of definable cause, and lack of family history falls clearly outside previously described categories. Accordingly, this type of hypertensive disease is presented as a new entity.
Report of CasesIdentical female infants were born to a 29-year-old white woman following a fullterm gestation complicated only by a third trimester pedal edema. The twins' sub¬ sequent course was remarkably similar.Case l.-The first-born developed gross hematuria on day 3. Results of physical ex¬ amination were normal and a flush blood pressure was 60 mm Hg. She was trans¬ ferred to Milwaukee Children's Hospital where a hemogram, prothrombin time, and platelet count were normal. Her urine con¬ tained protein (4 + ) and was still grossly bloody. A urine culture was sterile and the blood urea nitrogen (BUN) level was 41 mg/100 ml. The kidneys were not visual¬ ized by intravenous pyelogram and no source of bleeding was found at cystoscopy. A retrograde pyelogram and a Downloaded From: http://archpedi.jamanetwork.com/ by a University of Calgary User on 05/27/2015